Perioperative management of pheochromocytoma: the heart of the issue

Pheochromocytoma is an endocrine tumor derived from the adrenal medulla and paraganglia. Pheochromocytoma presents with a wide spectrum of symptoms, from a silent adrenal mass to cardiac arrest. Perioperative management of pheochromocytoma is critical for preventing perioperative cardiovascular complications. Traditionally, perioperative management focuses on blood pressure control, which has generated considerable controversy. In this review, we suggest that perioperative management should focus more on treating subclinical and clinical pheochromocytoma-induced cardiomyopathy. We first describe the natural history of pheochromocytoma and illustrate that cardiomyopathy is present to various degrees in patients with pheochromocytoma. We then classify pheochromocytomas into 3 groups according to the risks of developing clinical cardiomyopathy. After going over perioperative physiological changes, we propose that the need for preoperative preparation depends on the risk level of the pheochromocytoma. We present the regimens for perioperative management, emphasizing that the goals of perioperative management should extend beyond blood pressure control and include improvement of cardiac function. Perioperative management in unique clinical situations is also discussed.